Article
Quantifying physical decline in juvenile neuronal ceroid lipofuscinosis (Batten disease).
Neurology - 15 Nov 2011
Kwon J M, Adams H, Rothberg P G, Augustine E F, Marshall F J, Deblieck E A, Vierhile A, Beck C A, Newhouse N J, Cialone J, Levy E, Ramirez-Montealegre D, Dure L S, Rose K R, Mink J W
Abstract excerpt
OBJECTIVE: To use the Unified Batten Disease Rating Scale (UBDRS) to measure the rate of decline in physical and functional capability domains in patients with juvenile neuronal ceroid lipofuscinosis (JNCL) or Batten disease, a neurodegenerative lysosomal storage disorder. We have evaluated the U...
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